Chilaiditi's Syndrome

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Copyright 2010, 2013

NORD is very grateful to Michael J Boros, MD, Munson Medical Center, for assistance in the preparation of this report.

Synonyms of Chilaiditi's Syndrome

No synonyms found.

Disorder Subdivisions

No subdivisions found.

General Discussion

Chilaiditi's syndrome is a rare condition in which a portion of the colon is abnormally located (interposed) in between the liver and the diaphragm. The diaphragm is the muscle that separates the chest cavity from the abdomen. Chilaiditi's syndrome can cause a variety of symptoms including abdominal pain, nausea, vomiting, and small bowel obstruction. The specific symptoms and presentation of Chilaiditi's syndrome can vary greatly from one person to another. The cause of Chilaiditi's syndrome is not fully understood.

In most cases, interposition of a portion of colon between the liver and diaphragm does not cause symptoms (asymptomatic) and is often an incidental finding in the elderly. When no symptoms are present, this clinical finding is referred to as Chilaiditi's sign. In rare cases, symptoms do develop; these cases are referred to as Chilaiditi's syndrome.

Symptoms

The symptoms of Chilaiditi's syndrome may vary from one person to another. The presentation and specific symptoms that develop can be significantly different. Chronic, recurrent episodes of abdominal pain are a common finding. Abdominal pain may be mild and come and go (intermittent). However, abdominal pain can be severe and force affected individuals to go to the emergency room.

Additional symptoms can occur with Chilaiditi's syndrome. These symptoms can occur together in a wide variety of different combinations. Affected individuals will not have all of these symptoms and some affected individuals will not have any of these symptoms.

Additional symptoms that have been reported in Chilaiditi's syndrome include nausea, vomiting, constipation, indigestion (dyspepsia), abnormal twisting of the intestines (volvulus) causing obstruction, abdominal swelling (distention), difficulty swallowing (dysphagia), and tenderness in the upper, central area of the abdomen (epigastric region). In some cases, Chilaiditi's syndrome has been associated with breathing problems (respiratory distress).

Causes

The exact cause of Chilaiditi's syndrome is unknown. The condition occurs with greater frequency in individuals with chronic lung disease, scarring of the liver (cirrhosis), and the accumulation of fluid within the abdominal cavity (ascites). Ascites can be associated with a wide variety of medical conditions.

Additional predisposing factors include abnormal elongation of the colon or abnormal looseness (laxity) of certain ligaments of the colon and liver. Ligaments are tough, fibrous bands of tissue that primarily serve to connect or support structures within the body.

According to the medical literature, other factors may play a role in the development of Chilaiditi's syndrome in some cases including reduced liver volume, paralysis of the motor nerve of the diaphragm (phrenic nerve palsy) and obesity.

Affected Populations

Chilaiditi's syndrome affects males and females in equal numbers. It is more common in older adults, but can occur at any age and has been reported in children. The incidence of Chilaiditi's syndrome is unknown. One published report estimated the incidence at .025-.28 percent of the general population. Chilaiditi's syndrome was first described in the medical literature in 1910 by a Greek radiologist named Demetrius Chilaiditi.

Related Disorders

Symptoms of the following disorders can be similar to those of Chilaiditi's syndrome. Comparisons may be useful for a differential diagnosis.

Pneumoperitoneum is the abnormal presence of gas or air within the abdominal cavity. Pneumoperitoneum can have a variety of different causes. The specific symptoms that occur depend upon the cause. Chilaiditi's syndrome can potentially be mistaken for pneunoperitoneum, which can result in unnecessary surgery.

Standard Therapies

Diagnosis
A diagnosis of Chilaiditi's syndrome is made based upon imaging (radiographic) confirmation of the abnormal positioning of the colon and the occurrence of associated symptoms. Such imaging techniques may include chest and abdominal x- rays, ultrasounds, or computerized tomography (CT) scanning.

Treatment
The treatment of Chilaiditi's syndrome is directed toward the specific symptoms that are apparent in each individual. Some affected individuals may not require any therapy. The removal of pressure from the intestines (bowl decompression) has alleviated symptoms in some cases.

In some cases, surgical intervention may be required. Surgical techniques that have been used to treat individuals with Chilaiditi's syndrome include the removal of a portion of the colon (transverse colectomy or right hemicolectomy) or the anchoring of a displaced liver to the abdominal wall (hepatopexy).

Investigational Therapies

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